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1.
Am J Dermatopathol ; 42(1): 24-28, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31169526

RESUMO

Specific cutaneous involvement in Hodgkin lymphoma is rare. In cutaneous lesions, the diagnosis is usually based on the recognition of diagnostic Reed-Sternberg cells and its variants. In nodal Hodgkin lymphoma, so-called mummified cells (cells with condensed cytoplasm and pyknotic eosinophilic or basophilic nuclei) are often seen. They are sometimes conspicuous and easy to recognize, thus serving as a clue to the diagnosis. Our objective was to study cases of cutaneous Hodgkin lymphoma to identify the occurrence of mummified cells. We studied 12 patients (4 women and 8 men; age range 23-80 years). In 6 patients, cutaneous and extracutaneous disease was identified almost simultaneously; in 4 patients, lymph node disease preceded cutaneous involvement; and in the remaining 2 patients, the skin lesions were the presenting sign, whereas lymph node involvement occurred later. Histopathological, immunohistochemical, and molecular-genetic studies, including rearrangements for TCR, IgH genes, and PCR for EBV, were performed. Cutaneous biopsy specimens revealed either a multinodular or diffuse infiltrate, included small lymphocytes, eosinophils, plasma cells, and macrophages, but in all cases, diagnostic Reed-Sternberg cells and its variants were identified. Mummified cells were detected in 9 cases, either as occasional scattered mummified cells often requiring a search (6 cases) or being conspicuous, grouped and therefore easily identified (3 cases). Immunohistochemically, in all 7 cases studied, mummified cells were positive for both CD30 and CD15. It is concluded that mummified cells are encountered in a majority of cases of cutaneous Hodgkin lymphoma.


Assuntos
Doença de Hodgkin/patologia , Neoplasias Cutâneas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
2.
J Am Acad Dermatol ; 80(2): 341-363, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30321581

RESUMO

Dermoscopy is increasingly used by clinicians (dermatologists, family physicians, podiatrists, doctors of osteopathic medicine, etc) to inform clinical management decisions. Dermoscopic findings or images provided to pathologists offer important insight into the clinician's diagnostic and management thought process. However, with limited dermoscopic training in dermatopathology, dermoscopic descriptions and images provided in the requisition form provide little value to pathologists. Most dermoscopic structures have direct histopathologic correlates, and therefore dermoscopy can act as an excellent communication bridge between the clinician and the pathologist. In the first article in this continuing medical education series, we review dermoscopic features and their histopathologic correlates.


Assuntos
Carcinoma Basocelular/patologia , Carcinoma de Células Escamosas/patologia , Dermoscopia/métodos , Neoplasias Cutâneas/patologia , Adulto , Idoso , Biópsia por Agulha , Carcinoma Basocelular/diagnóstico , Carcinoma de Células Escamosas/diagnóstico , Diagnóstico Diferencial , Educação Médica Continuada , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Sensibilidade e Especificidade , Neoplasias Cutâneas/diagnóstico
3.
J Am Acad Dermatol ; 80(2): 365-377, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30321580

RESUMO

Multiple studies have shown that dermoscopy increases the sensitivity and specificity for the detection of skin cancers compared with examination by the naked eye. Dermoscopy can also lead to the detection of thinner and smaller cancers. In addition, dermoscopy leads to the more precise selection of lesions requiring excision. In essence, dermoscopy helps clinicians differentiate benign from malignant lesions through the presence or absence of specific dermoscopic structures. Therefore, because most dermoscopic structures have direct histopathologic correlates, dermoscopy can allow the prediction of certain histologic findings present in skin cancers, thus helping select management and treatment options for select types of skin cancers. Visualizing dermoscopic structures in the ex vivo specimens can also be beneficial. It can improve the histologic diagnostic accuracy by targeted step-sectioning in areas of concern, which can be marked by the clinician before sending the specimen to the pathologist, or by the pathologist on the excised specimen in the laboratory. In addition, ex vivo dermoscopy can also be used to select tumor areas with genetic importance because some dermoscopic structures have been related to mutations with theragnostic relevance. In the second article in this continuing medical education series, we review the impact of dermoscopy on the diagnostic accuracy of skin cancer, how dermoscopy can affect the histopathologic examination, and which dermoscopic features may be more relevant in terms of histologic and genetic prediction.


Assuntos
Carcinoma Basocelular/diagnóstico , Carcinoma de Células Escamosas/diagnóstico , Dermoscopia/métodos , Melanoma/diagnóstico , Neoplasias Cutâneas/diagnóstico , Biópsia por Agulha , Carcinoma Basocelular/patologia , Carcinoma de Células Escamosas/patologia , Diagnóstico Diferencial , Educação Médica Continuada , Feminino , Humanos , Imuno-Histoquímica , Masculino , Melanoma/patologia , Estadiamento de Neoplasias , Sensibilidade e Especificidade , Neoplasias Cutâneas/patologia
4.
Am J Dermatopathol ; 41(4): 281-285, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30398985

RESUMO

Syringocystadenoma papilliferum (SCAP) is a benign tumor most commonly located on the head and neck area often associated with nevus sebaceus. In its usual location, the human papillomavirus (HPV) DNA and mutations in the RAS/mitogen-activated protein kinase signaling pathway have been detected in SCAP. We studied 16 cases of SCAP in the anogenital areas and buttock where this neoplasm is rare and attempted to find out whether SCAP in these sites have different histopathological and molecular biological features. It seems that there is no significant difference between the morphology of anogenital SCAP and SCAP in other locations. Several tumors in our cohort demonstrated features resembling those seen in warts, but HPV DNA was not found in these lesions. On the contrary, we identified DNA of HPV high-risk types in some tumors without HPV-related morphology. Our study confirms the role of HRAS and BRAF V600 mutations in the pathogenesis of SCAP, including SCAP in the anogenital areas and buttock.


Assuntos
Infecções por Papillomavirus/epidemiologia , Proteínas Proto-Oncogênicas B-raf/genética , Proteínas Proto-Oncogênicas p21(ras)/genética , Neoplasias das Glândulas Sudoríparas/genética , Adenomas Tubulares de Glândulas Sudoríparas/genética , Adenomas Tubulares de Glândulas Sudoríparas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Canal Anal/patologia , Nádegas/patologia , Feminino , Neoplasias dos Genitais Femininos/genética , Neoplasias dos Genitais Femininos/patologia , Neoplasias dos Genitais Femininos/virologia , Neoplasias dos Genitais Masculinos/genética , Neoplasias dos Genitais Masculinos/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Papillomaviridae , Neoplasias das Glândulas Sudoríparas/patologia , Neoplasias das Glândulas Sudoríparas/virologia , Adenomas Tubulares de Glândulas Sudoríparas/virologia , Adulto Jovem
5.
Case Rep Dermatol ; 10(2): 162-168, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30022936

RESUMO

Decorative tattooing is a procedure in which exogenous pigment and/or dye is introduced into the dermis with the aim of creating a permanent skin decoration. The increasing prevalence of tattooed individuals leads to more reported tattoo-related complications. Pseudolymphomatous reaction is a benign reactive proliferation of lymphocytes that may uncommonly occur secondary to tattooing. We describe the clinical, histological, and molecular aspects of a pseudolymphomatous reaction to red tattoo pigment.

6.
Am J Dermatopathol ; 40(7): 479-485, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29924747

RESUMO

Sebaceous neoplasms with an organoid pattern (rippled, labyrinthine/sinusoidal, carcinoid-like, and petaloid) are rare. Previous studies suggested that the above patterns likely represent variations along a morphological continuum. The objectives of this study were to (1) validate this proposition by studying a large number of cases, (2) determine whether there are specific associations with clinical features, (3) establish their frequency, and (4) determine whether they have any association with Muir-Torre syndrome. Fifty-seven sebaceous neoplasms (54 sebaceomas and 3 sebaceous carcinomas) with organoid growth patterns were studied. These occurred in 36 men and 18 women (sex unknown in 3), with ages at diagnosis ranging from 22 to 89 years (mean, 63 years). All patients presented with a solitary nodule (mean size, 11 mm) on the head and neck area. Of the 57 tumors, 24 manifested a single growth pattern, 23 had a combination of 2 patterns, and 10 a combination of 3 patterns, indicating that these patterns are part of a morphological continuum of changes. The carcinoid-like pattern was the most frequent in the "monopatterned" neoplasms (13 cases), whereas the labyrinthine/sinusoidal pattern comprised most of the "polypatterned" lesions, in which various combinations occurred. Immunohistochemically, mismatch repair protein deficiency was detected in 3 of the 22 cases studied, whereas 5 of the 33 patients with available follow-up had an internal malignancy/premalignancy. In conclusion, sebaceous neoplasms with organoid growth patterns are predominantly sebaceomas having a predilection for the scalp, occurring as solitary lesions in elderly patients (male to female ratio of 2:1). Such patterns are expected to be found in a quarter of sebaceomas. In most cases, more than one of the organoid patterns is present. These lesions do not appear to be associated with internal malignancy or mismatch repair deficiency in most cases. However, confirmation of the absence of any significant association with Muir-Torre syndrome syndrome will require genetic studies.


Assuntos
Neoplasias das Glândulas Sebáceas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Reparo de Erro de Pareamento de DNA/genética , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Muir-Torre/complicações , Neoplasias das Glândulas Sebáceas/etiologia , Adulto Jovem
7.
Case Rep Dermatol ; 10(2): 133-137, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29928202

RESUMO

Basal cell carcinoma (BCC) is the most common cancer in humans worldwide. Many highly specific dermoscopic criteria for BCC are well established in the literature. On the contrary, other malignant or benign skin tumors may mimic BCC by exhibiting similar or even the same dermoscopic features and therefore obscuring the diagnosis of BCC in certain situations. We herein report a case of BCC with dermoscopic features of both BCC and desmoplastic trichoepithelioma (DT). We would like to remind of the often neglected differential diagnosis of DT in a lesion with arborizing vessels and otherwise unusual dermoscopic presentation.

8.
Semin Cutan Med Surg ; 37(1): 24-29, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29719017

RESUMO

Cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ LPD) are the second most common form of cutaneous T-cell lymphoma. CD30+ LPD include lymphomatoid papulosis, primary cutaneous anaplastic large-cell lymphoma, and borderline lesions. Despite expression of CD30 by the neoplastic cells as the hallmark of these disorders, they differ in their clinical presentation and histological features as well as the course, the prognosis, and consecutively in the treatment. Diagnosis of CD30+ LPD and distinction from the broad spectrum of differential diagnoses essentially depends on clinicopathologic correlation as well as the results of staging examinations. Although the histological findings indicate a high-grade lymphoma, CD30+ LPD in most cases have a favorable prognosis. Recent advances in targeted therapy have led to new therapeutic approaches to CD30+ LPDs. This review describes the clinicopathologic features of CD30+ LPDs, their differential diagnoses, the treatment, and the role of CD30 as a diagnostic marker and therapeutic target.


Assuntos
Papulose Linfomatoide/diagnóstico , Neoplasias Cutâneas/diagnóstico , Linfócitos T , Humanos , Papulose Linfomatoide/patologia , Papulose Linfomatoide/terapia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia
9.
Acta Derm Venereol ; 98(9): 835-841, 2018 Oct 10.
Artigo em Inglês | MEDLINE | ID: mdl-29693696

RESUMO

Rare T- or NK-cell lymphomas with cutaneous manifestation may display a highly aggressive clinical course and major diagnostic/therapeutic challenges. This report describes our experiences with different lymphomas of this rare category and the therapeutic options used. This retrospective, descriptive, monocentric, cross-sectional case study, identified 4 rare aggressive T-/NK-cell lymphomas with manifestation in the skin, which were diagnosed in a tertiary care centre over a period of 4 years. Two patients had an Epstein-Barr virus-associated extranodal NK/T-cell lymphoma and 2 patients had a primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma. Concomitant extracutaneous involvement was observed in 2 of all 4 patients. Two patients had fulminant disease progression and resistance to chemotherapy. Two patients underwent allogeneic haematopoietic stem cell transplantation, which resulted in one complete remission and one partial remission. This report emphasizes the importance of an early diagnostic work-up and a prompt aggressive therapeutic approach.


Assuntos
Linfoma Extranodal de Células T-NK/patologia , Linfoma Cutâneo de Células T/patologia , Neoplasias Cutâneas/patologia , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/uso terapêutico , Estudos Transversais , Progressão da Doença , Resistencia a Medicamentos Antineoplásicos , Detecção Precoce de Câncer , Evolução Fatal , Feminino , Transplante de Células-Tronco Hematopoéticas , Herpesvirus Humano 4/isolamento & purificação , Humanos , Linfoma Extranodal de Células T-NK/imunologia , Linfoma Extranodal de Células T-NK/terapia , Linfoma Extranodal de Células T-NK/virologia , Linfoma Cutâneo de Células T/imunologia , Linfoma Cutâneo de Células T/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/terapia , Neoplasias Cutâneas/virologia , Suíça , Linfócitos T Citotóxicos/imunologia , Linfócitos T Citotóxicos/patologia , Fatores de Tempo , Resultado do Tratamento
10.
Neoplasia ; 20(2): 218-225, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29331888

RESUMO

A deepened understanding of the cellular and molecular processes in the tumor microenvironment is necessary for the development of precision immunotherapy (IT). We simultaneously investigated CD3, PDL1, and IDO by immunohistochemistry in paired biopsies from various organs of 43 metastatic melanoma patients treated with IT and targeted therapy (TT). Intraindividual biopsies taken after a period of weeks to months demonstrate discordant results in 30% of the cases. Overlap of IDO and PDL1 increased after therapy. IT only marginally impacted PDL1 expression over time in contrast to TT. Standardized repeated assessments of multiple immune markers in repeated biopsies will generate detailed insights in melanoma's immune evolution and adaption during therapies and might be used to support treatment decisions.


Assuntos
Antineoplásicos/uso terapêutico , Antígeno B7-H1/metabolismo , Biomarcadores Tumorais/metabolismo , Imunoterapia , Indolamina-Pirrol 2,3,-Dioxigenase/metabolismo , Melanoma/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Antígeno B7-H1/antagonistas & inibidores , Antígeno B7-H1/imunologia , Biomarcadores Tumorais/antagonistas & inibidores , Biomarcadores Tumorais/imunologia , Feminino , Seguimentos , Humanos , Indolamina-Pirrol 2,3,-Dioxigenase/antagonistas & inibidores , Indolamina-Pirrol 2,3,-Dioxigenase/imunologia , Estudos Longitudinais , Masculino , Melanoma/tratamento farmacológico , Melanoma/imunologia , Melanoma/secundário , Pessoa de Meia-Idade , Terapia de Alvo Molecular , Prognóstico , Taxa de Sobrevida , Adulto Jovem
12.
J Cutan Pathol ; 45(1): 23-28, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29023827

RESUMO

BACKGROUND: Tumor necrosis factor α (TNFα) blocking drugs are in use for a wide range of autoimmune disorders. In up to 5% of patients, this class of drugs produces puzzling cutaneous side effects that are the subject of this investigation, namely psoriasiform and eczema-like skin inflammation. These side effects can occur after any time of treatment and regardless of the underlying disorders. The exact pathophysiology is as yet unknown. METHODS: A total of 33 patients (19 female, average age 52 years) who had a cutaneous reaction to infliximab, adalimumab or etanercept were included. The type of inflammatory reaction was determined, and the corresponding cytokine expression was evaluated by immunohistochemistry for TNFα, IL-1ß, IL-22, IL-6, IL-17A, IL33, IL-8 and IL-36α (semi-quantitative grading system from - to ++++). In addition, RNA expression levels of IL-17A and TNFα were confirmed by quantitative real-time PCR. RESULTS: IL-17A (P < .039) and TNFα (P < .008) were expressed at significantly higher levels in psoriasis or pustular-like reactions (PPR) compared to eczematous-like reactions (ELR). There was no significant difference in the expression of IL-1ß, IL-22, IL-6, IL-33, IL-8 and IL-36α between PPR and ELR. CONCLUSION: TNFα and IL-17A are both cytokines known to be involved in psoriasis but less so in non-psoriasiform dermatitis or eczema. Therefore, their overexpression in PPR is plausible and suggests that the pathogenesis of PPR mirrors at least in part those of psoriasis. Further investigations will define the exact role of these cytokines in rare cutaneous side effects of anti-TNFα therapy. Our results suggest that IL-17A inhibition could be a therapeutic option in patients with anti-TNF induced psoriasis.


Assuntos
Antirreumáticos/efeitos adversos , Erupção por Droga/metabolismo , Eczema/induzido quimicamente , Interleucina-17/biossíntese , Psoríase/induzido quimicamente , Fator de Necrose Tumoral alfa/biossíntese , Adalimumab/efeitos adversos , Adulto , Idoso , Erupção por Droga/etiologia , Erupção por Droga/patologia , Etanercepte/efeitos adversos , Feminino , Humanos , Infliximab/efeitos adversos , Masculino , Pessoa de Meia-Idade , Fator de Necrose Tumoral alfa/antagonistas & inibidores
13.
Dermatology ; 233(4): 268-276, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29130957

RESUMO

BACKGROUND/AIM: Pyoderma gangrenosum (PG) is a rare, neutrophilic dermatosis often associated with an underlying disease, and clinical data or larger studies are rare. METHODS: In this retrospective study, disease characteristics, clinical manifestations, and treatment response were evaluated in a Swiss cohort of PG patients. RESULTS: In participating centers, 34 cases (21 females) of PG were analyzed based on clinical and histological presentation between 2002 and 2012. The mean age at diagnosis was 61.2 years; 50% of the patients experienced only 1 episode of PG. In 13 cases (out of 20), recurrences occurred during PG therapy; 64.1% showed only 1 lesion simultaneously. The predominant localization was the lower limb (67%). The lesions were disseminated in 26.6%. At the time of diagnosis or recurrence, the mean diameter was 37.6 mm and the mean ulcer size was 10.3 cm2. C-reactive protein (CRP) was elevated in 73.2%; leukocytosis was present in 58.9% and neutrophilia in 50.9%. At least 1 associated comorbidity was present in 85% (the most prominent being cardiovascular disease). The most often used systemic treatments were steroids (68.3%), cyclosporine A (31.7%), dapsone (31.7%), and infliximab (13.3%), and the most often used topicals were tacrolimus 0.1% (48.3%) and corticosteroids (35%). PG healed completely at discharge in 50.8%. The average time to diagnosis was 8 months, and the mean duration to healing was 7.1 months. CONCLUSION: PG is a difficult-to-diagnose skin disease. Here, markers for inflammation such as CRP, leukocytosis, and neutrophilia were elevated in 50-73% of the PG patients.


Assuntos
Fármacos Dermatológicos/administração & dosagem , Pioderma Gangrenoso/epidemiologia , Pele/patologia , Administração Cutânea , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Feminino , Seguimentos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/tratamento farmacológico , Estudos Retrospectivos , Índice de Gravidade de Doença , Suíça/epidemiologia , Adulto Jovem
14.
Eur J Pediatr ; 176(12): 1663-1668, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28944414

RESUMO

Little is known about the spectrum of pediatric skin disorders requiring biopsy/excision, their indication, impact on further management, and the accuracy of clinical diagnosis. We aimed to address these questions in the patient population seen at our Swiss University referral center for Pediatric Dermatology and Plastic Surgery. All skin biopsies/excisions performed in patients aged ≤ 16 years over a period of 2 years were retrospectively analyzed. A total of 506 samples were included. The majority of biopsies/excisions (n = 413, 82%) was performed for tumors, cysts, and hamartomas and 18% for other skin conditions. Malignant tumors were found in 12 samples (2%) from four patients. In 121 (24%) patients, the histopathology had an important impact on patient management. In 80 (16%) cases, the pathology did not match with the clinical diagnosis. In 382 (75%) cases, excision was the treatment of choice. Of these, the indication for surgery was based on patient's request in 181 (47%) cases. CONCLUSION: Surgical interventions for pediatric skin disorders are performed for diagnostic and therapeutic reasons. In this cohort, histopathology was essential for treatment in one quarter of cases. Skin tumors, cysts, and hamartomas often require excision during childhood, with families' request and esthetic considerations playing an important role. What is Known: • The spectrum of pediatric skin conditions has been studied in outpatient, inpatient, and emergency settings. • In contrast, no data exist on the spectrum of pediatric skin disorders undergoing biopsy/excision specifically. What is New: • We analyze biopsies/excisions in children, focusing on diagnosis, indication, and impact on patient management. • Surgical interventions for skin disorders in children are often performed for tumors and hamartomas with esthetic considerations playing a relevant role. If used for diagnostic purposes, they are often performed to confirm or rule out severe skin disease.


Assuntos
Dermatopatias/diagnóstico , Pele/patologia , Adolescente , Biópsia , Criança , Pré-Escolar , Procedimentos Cirúrgicos Dermatológicos , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Dermatopatias/patologia , Dermatopatias/cirurgia
15.
Med Mycol Case Rep ; 18: 8-11, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28761802

RESUMO

A patient with tinea incognita is presented together with a review of the literature of figurate erythema. Figurate lesions are emblematic for dermatology and perhaps the most picturesque efflorescences. The differential diagnosis can be broad and sometimes challenging. Many clinical entities with resembling primary and secondary efflorescences have to be considered as differentials and can be due to anti-infectious, paraneoplastic, allergic, autoimmune or other immune reactions.

16.
Am J Surg Pathol ; 41(8): 1053-1058, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28614205

RESUMO

To determine whether a subset of primary extramammary Paget disease (EMPD) may originate in anogenital mammary-like glands (AGMLG), the authors studied 181 specimens of EMPD, detailing alterations in AGMLG. The latter were identified in 33 specimens from 31 patients. All patients were women, ranging in age from 38 to 93 years (median, 65 y). In all cases, lesions involved the vulva and in 1 patient the perianal skin was affected. Histopathologically, AGMLG manifested changes identical to columnar cell change (CCC) (87.1%), usual ductal hyperplasia (22.6%), columnar cell hyperplasia (CCH) (9.7%), oxyphilic (apocrine) metaplasia (6.5%), and atypical duct hyperplasia (3.2%). Four cases (12.9%), in addition to intraepidermal carcinoma, harbored invasive carcinoma. In all 4 of these, AGMLG displayed a range of alterations including ductal carcinoma in situ, CCC, and CCH. Three further cases (9.7%) showed ductal carcinoma in situ without any definite invasive carcinoma. Colonization of AGMLG by neoplastic Paget cells was noted in 6 cases. As CCC and CCH may be encountered in normal AGMLG, these alterations are unlikely to play a significant role in the pathogenesis of the disease. However, by analogy with mammary Paget disease, rare cases of primary EMPD may originate in AGMLG with a subsequent upward migration of the neoplastic cells into the epidermis and possible later breach through the basal membrane. Usual ductal hyperplasia and atypical duct hyperplasia can then be regarded as earlier precursor lesions, linking both ends of the spectrum.


Assuntos
Canal Anal/patologia , Neoplasias do Ânus/etiologia , Neoplasias do Ânus/patologia , Doença de Paget Extramamária/etiologia , Doença de Paget Extramamária/patologia , Vulva/patologia , Neoplasias Vulvares/etiologia , Neoplasias Vulvares/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Pessoa de Meia-Idade
17.
Case Rep Dermatol ; 9(1): 74-79, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28611626

RESUMO

Angioimmunoblastic T-cell lymphoma (AITCL) is a rare, aggressive lymphoma which derives from follicular helper T cells, commonly affecting the elderly population. It accounts for 2% of all non-Hodgkin lymphomas, with a reported 5-year overall survival rate of less than 30%. Very often, the clinical picture of AITCL encompasses systemic symptoms such as generalized lymphadenopathy, hepatosplenomegaly, skin rash, anemia, and polyclonal hypergammaglobulinemia. Here we report on the case of a female patient who presented with clinical features resembling drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) prior to the definitive diagnosis of AITCL. The index of suspicion for cutaneous manifestations of lymphoma, and especially AITCL, must be high, particularly in atypical clinical courses of drug eruptions or if skin lesions relapse and are refractory to standard high-dose systemic corticosteroids.

18.
Am J Surg Pathol ; 41(7): 998-1004, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28504999

RESUMO

Programmed death ligand 1 (PD-L1) is expressed by 20% to 57% of systemic diffuse large B cell lymphomas (DLBCLs). PD-L1 expression in primary cutaneous DLBCL (pcDLBCL) has not been studied so far. Sixteen paraffin-embedded tissue samples of pcDLBCL (13 leg type [LT], 3 others [OT]) were investigated for PD-1, PD-L1, and CD33 expression and the cellular composition of the tumor microenvironment, focusing on myeloid-derived suppressor cells (MDSCs) and tumor-associated macrophages. Membrane-bound PD-L1 expression by the tumor cells was observed in all samples, albeit to a variable extent (19.9%). As expected, most DLBCL-LT (10 cases) were classified as activated B cell like type, with a higher PD-L1 score (21.9%) compared with that of the germinal center B cell like type (7.7%). The surrounding infiltrate consisted predominately of CD163(+) M2 rather than CD68(+) macrophages (CD68:CD163=1:4 to 6). Moreover, a considerable proportion of CD33(+) MDSCs with PD-L1 coexpression was admixed. Tumor cells expressed CD33 to variable degrees (2% to 60%). The number of MDSCs or M2 macrophages did not correlate with pcDLBCL subtypes LT or OT. T cells were only a minor component of the tumor microenvironment. We propose that PD-L1(+) tumor cells and PD-L1(+) MDSCs shield the tumor against PD-1(+) tumor-infiltrating lymphocytes, consequently leading to inhibition and diminution of tumor-infiltrating lymphocytes. Moreover, we found a polarization to M2 macrophages, which may contribute to the poor prognosis of DLBCL patients. Thus, targeting of tumor cells and MDSCs using anti-PD-1/anti-PD-L1 or anti-CD33 antibodies might be a worthwhile new approach to treat this aggressive form of cutaneous B-cell lymphoma.


Assuntos
Antígeno B7-H1/metabolismo , Biomarcadores Tumorais/metabolismo , Linfoma Difuso de Grandes Células B/patologia , Receptor de Morte Celular Programada 1/metabolismo , Lectina 3 Semelhante a Ig de Ligação ao Ácido Siálico/metabolismo , Neoplasias Cutâneas/patologia , Microambiente Tumoral , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Feminino , Humanos , Linfoma Difuso de Grandes Células B/metabolismo , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias Cutâneas/metabolismo
19.
Am J Dermatopathol ; 39(4): 275-278, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28323778

RESUMO

The histopathological diagnosis of periocular sebaceous carcinoma can be difficult in poorly differentiated cases showing few mature sebocytes. The authors examined 50 periocular sebaceous carcinomas from 46 patients to determine the frequency of 2 features seen in this neoplasm, namely cells with squared-off nuclei and so-called "appliqué" pattern (peritumoral subnecrosis of peripherally located neoplastic cells). Neoplastic cells with squared-off nuclei were found in varying numbers in both the intraepithelial and dermal (invasive) components in all neoplasms, whereas the appliqué pattern was observed in a third of the cases. It is concluded that these features, taken together, may serve as a clue for the diagnosis of periocular sebaceous carcinoma.


Assuntos
Adenocarcinoma Sebáceo/patologia , Neoplasias Oculares/patologia , Neoplasias das Glândulas Sebáceas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
20.
Dermatol Ther (Heidelb) ; 7(1): 167-174, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28168623

RESUMO

INTRODUCTION: Solid facial edema (SFE) is a rare complication of acne vulgaris. To examine the clinical features of acne patients with solid facial edema, and to give an overview on the outcome of previous topical and systemic treatments in the cases so far published. METHODS: We report two cases from Switzerland, both young men with initially papulopustular acne resistant to topical retinoids. RESULTS: Both cases responded to oral isotretinoin, in one case combined with oral steroids. Our cases show a strikingly similar clinical appearance to the cases described by Connelly and Winkelmann in 1985 (Connelly MG, Winkelmann RK. Solid facial edema as a complication of acne vulgaris. Arch Dermatol. 1985;121(1):87), as well as to cases of Morbihan's disease that occurs as a rare complication of rosacea. CONCLUSION: Even 30 years after, the cause of the edema remains unknown. In two of the original four cases, a potential triggering factor was identified such as facial trauma or insect bites; however, our two patients did not report such occurrencies. The rare cases of solid facial edema in both acne and rosacea might hold the key to understanding the specific inflammatory pattern that creates both persisting inflammation and disturbed fluid homeostasis which can occur as a slightly different presentation in dermatomyositis, angioedema, Heerfordt's syndrome and other conditions.

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